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SPRENGEL DEFORMITY OF THE SHOULDER

Clinical presentation
Sprengel deformity is usually noticed at birth and has both cosmetic and functional implications. The elevated scapula is visually noticeable and there is an associated restriction in the motion of the scapula and glenohumeral joint.
Classification
The Cavendish classification 2,6 is one method used for grading:
grade I
very mild deformity is observed
when covered with clothes the deformity is almost invisible
grade II
the deformity is still mild but appears as a bump
the superomedial portion of the high scapula is convex, forming a bump
grade III
moderate deformity with 2-5 cm of visible elevation of the affected shoulder
grade IV
severe deformity with >5 cm elevation of the affected shoulder, accompanied by neck webbing
Pathology
The abnormality results from failure of caudal migration of the scapula during early fetal development.
Associations
Sprengel deformities usually coexist with other congenital abnormalities, particularly those involving the vertebrae and ribs. An omovertebral bar (fibrous, cartilaginous and/or osseous connection between the scapula and cervical spine) is often present.
It is also commonly associated with hypoplasia or atrophy of regional muscles, and these associated features can cause further misshaping of the shoulder and limitation of shoulder movement.
Patients with Sprengel deformity often have one or more of the following abnormalities and conditions:
Klippel-Feil syndrome
spina bifida
kyphoscoliosis
torticollis
underdevelopment of clavicle or humerus
These possible co-existing anomalies need to be looked for in any patient presenting with Sprengel deformity.
Radiographic features
Plain radiograph
The affected scapula is elevated and rotated, with the inferior angle directed laterally.
The radiographic Rigault classification 
grade I: superomedial angle lower than T2 but above T4 transverse process
grade II: superomedial angle located between C5 and T2 transverse process
grade III: superomedial angle above C5 transverse process
CT
CT with 3D reconstruction is being used to evaluate omovertebral connection and scapula dysplasia and malpositioning. It can be used in preoperative planning.
MRI
There may be a role in MRI to assess omovertebral connection
Treatment and prognosis
Surgery is performed to improve cosmetic and functional disability. It is generally considered for patients between 3 and 8 years of age who have moderate to severe disability (or a Cavendish score of 3-4) 1.
Two of the most used surgical methods are the “Woodward” procedure and the “modified-Green” procedure with good functional and cosmetic outcome.
History and etymology
It is named after Otto Gerhard Karl Sprengel (1852-1915), a German surgeon who described four cases in 1891.

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Updated: October 25, 2017 — 7:25 am

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